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MINISTRY OF HEALTH OF UKRAINE
VINNYTSIA NATIONAL MEDICAL UNIVERSITY
NAMED AFTER M.I.PIROGOV
NEUROLOGY DEPARTMENT
Stomatology Faculty
Lesson #5
Cerebrum. Cerebral Cortex. Higher Cortical
Functions. Consciousness.
1. Goals:
1.1.
To study the Anatomical fundamentals of the Cerebral Gray and
White Matter.
1.2.
To study the Functional peculiarities of the Higher Brain Centers and
their anatomical localisations.
1.3.
To study the Neurological fundamentals of the Consciousness and its
changes in different Neurological conditions.
2. Basic questions:
2.1. Anatomical Fundamentals:
2.1.1. Cerebral cortex. Cortical Layers .
2.1.2. Cerebral White Matter. Fibers.
2.2. Functional Localisations in the Cerebral Cortex:
2.2.1. Primary Somatosensory and Motor Cortical Areas.
2.2.2. Primary Visual Cortex.
2.2.3. Primary Gustatory Cortex.
2.2.4. Primary Vestibular Cortex.
2.2.5. Association Areas.
2.3. Higher Cortical Functions and Their Impairment by Cortical
Lesions
2.3.1. Aphasia.
2.3.2. Apraxia.
2.3.3. Agnosia.
2.4. Consciousness.
2.4.1. Morphological and functional substrate of Consciousness.
2.4.2. Impaired Consciousness. Coma. Glasgow Coma Scale.
Literature:
Mathias Baehr, M.D., Michael Frotscher, M.D. Duus’ Topical Diagnosis in
Neurology. – P.349-400
Mark Mumenthaler, M.D., Heinrich Mattle, M.D. Fundamentals of Neurology.
– P.39-42.
Histological Organization of the Cerebral
Cortex
The folded surface of the brain is made up of the gray matter of
the cerebral cortex, which is gray because of the very high density of
neurons within it. The cortex varies in thickness from 1.5mm (visual
cortex) to 4.55mm (precentral gyrus); it is generally thicker on the crown
of a gyrus than in the depths of the neighboring sulci.
The internal structure of the six-layered isocortex is depicted in
Fig. 9.12. In an anatomical section perpendicular to the brain surface, the
following layers can be distinguished, from outside to inside (i.e., from
the pial surface to the subcortical white matter).
1. Molecular layer (zonal layer). This layer is relatively poor in
cells. In addition to the distal dendritic trees (apical tuft) of lower-lying
pyramidal cells and the axons that make synaptic contact with them, this
layer contains mostly small neurons (Cajal-Retzius cells), whose
dendrites run tangentially within the layer. The
2. External granular layer. This layer contains many granule
cells (“nonpyramidal cells”) and a few pyramidal cells whose dendrites
branch out both within the external granular layer and upward into the
molecular layer. The nonpyramidal cells are mostly GABAergic
inhibitory neurons, while the pyramidal cells are excitatory and use
glutamate as their neurotransmitter.
3. External pyramidal layer. As its name implies, this layer
contains many pyramidal cells, which, however, are smaller than those
of the deeper cortical layers. These cells are oriented with their bases
toward the subcortical white matter.
4. Internal granular layer. Like the external granular layer, this
layer contains many nonpyramidal cells. These granule cells mainly
receive afferent input from thalamic neurons by way of the
thalamocortical projection.
5. Internal pyramidal layer. This layer contains medium-sized
and large pyramidal cells. The largest cells of this layer (Betz cells) are
found only in the region of the precentral gyrus. The especially thickly
myelinated neurites of these cells form the corticonuclear and
corticospinal tracts.
6. Multiform layer. This layer of polymorph cells is subdivided
into an inner, less dense layer containing smaller cells, and an outer layer
containing larger cells.
Cerebral White Matter
Each hemisphere contains a large amount of subcortical white matter,
which is composed of myelinated nerve fibers of varying thickness and
neuroglia (mainly oligodendrocytes, the cells that form myelin sheaths).
The subcortical white matter is bounded by the cerebral cortex, the
lateral ventricles, and the striatum. Its nerve fibers are of three types:
1 Projection fibers
2 Association fibers
3 Commissural fibers
Projection Fibers
Projection fibers connect different parts of the central nervous
system with each other over long distances.
Association Fibers
The association fibers (Figs. 9.15 and 9.16) make up most of the
subcortical white matter. These fibers connect neighboring distant
cortical areas of the same hemisphere with each other. The cerebral
cortex is able to carry out its diverse associative and integrative functions
only because all of its functionally important areas are tightly
interconnected and neural impulses can travel easily from one cortical
area to another. These extensive fiber connections between cortical areas
may also be an important anatomical substrate for the partial recovery of
function often seen in the aftermath of cortical injury (e. g., after trauma
or stroke). Over time, as the individual practices the impaired activities,
performance may improve because the corresponding neural impulses
have been redirected along the remaining, intact pathways.
Commissural Fibers
Fibers linking cortical regions with their counterparts in the
opposite cerebral hemisphere are called commissural fibers (Fig. 9.16c,
d) and are found in the corpus callosum and the anterior commissure.
Functional Localization in the Cerebral
Cortex
A patho-anatomically oriented functional analysis of cortical
structures was supplemented, from 1870 onward, by experiments with
direct electrical or chemical stimulation of the cerebral cortex, both in
animals and in humans. Later techniques, including stereotaxy,
electroencephalography, and microelectrode recording of potentials
from individual neurons and nerve fibers, yielded ever more detailed
functional “maps” of the brain (Fig. 9.17). The original idea of the
“localizability” of brain function remains valid after a century and a half
of study, especially with respect to the primary cortical areas, described
further below.
Primary Cortical Fields
Primary Somatosensory and Motor Cortical Areas
Localization and function. The primary somatosensory cortex
(areas 3, 2, and 1, Fig. 9.18) roughly corresponds to the postcentral
gyrus of the parietal lobe and a portion of the precentral gyrus. It extends
upward onto the medial surface of the hemisphere, where it occupies the
posterior portion of the paracentral lobule. The primary somatosensory
cortex is responsible for the conscious perception of pain and
temperature as well as somatic sensation and proprioception, mainly
from the contralateral half of the body and face. Its afferent input is
derived from the ventral posterolateral and posteromedial nuclei of the
thalamus (Fig. 6.4, p. 266). Even though some sensory stimuli,
particularly painful stimuli, may already be vaguely perceived at the
thalamic level, more recise differentiation in terms of localization,
intensity, and type of stimulus cannot occur until impulses reach the
somatosensory cortex. The conscious perception of vibration and
position is not possible without the participation of the cortex.
The
primary
motor
cortex
(area 4) roughly
corresponds to the
precentral gyrus of
the frontal lobe,
including
the
anterior wall of the
central sulcus, and
extends
upward
into the anterior
portion
of
the
paracentral lobule
on
the
medial
surface
of
the
hemisphere.
The
fifth cortical layer
in area 4 contains
the characteristic
Betz
pyramidal
cells, which give
off the rapidly
conducting, thickly myelinated fibers of the pyramidal tract. Area 4 is
thus considered the site of origin of voluntary movement, sending motor
impulses to the muscles by way of the pyramidal tract and anterior horn
cells of the spinal cord. It receives afferent input from other areas of the
brain that participate in the planning and initiation of voluntary
movement, particularly the ventro-oral posterior nucleus of the thalamus,
the premotor areas 6 and 8, and the somatosensory areas.
A lesion of the primary somatosensory cortex impairs or
abolishes the sensations of touch, pressure, pain, and temperature, as
well as two-point discrimination and position sense, in a corresponding
area on the opposite side of the body (contralateral hemihypesthesia or
hemianesthesia).
A lesion in area 4 produces contralateral flaccid hemiparesis.
Additional damage of the adjacent premotor area and the underlying
fiber tracts is necessary to produce spastic hemiparesis, which reflects
the interruption of nonpyramidal as well as pyramidal pathways. Focal
epileptic seizures restricted to the somatosensory cortex are characterized
by repetitive motor phenomena, such as twitching, or by
paresthesia/dysesthesia on the opposite side of the body or face (motor or
sensory jacksonian seizures).
Primary Visual Cortex
Localization and retinotopy. The primary visual cortex
corresponds to area 17 of the occipital lobe (Figs. 9.17, 9.18). It is
located in the depths of the calcarine sulcus, and in the gyri immediately
above and below this sulcus on the medial surface of the hemisphere, and
it extends only slightly beyond the occipital pole. It is also called the
striate (“striped”) cortex because of the white stripe of Gennari, which is
grossly visible within it in a perpendicular anatomical section. The visual
cortex receives input by way of the optic radiation from the lateral
geniculate body, in orderly, retinotopic fashion: the visual cortex of one
side receives visual information from the temporal half of the ipsilateral
retina and the nasal half of the contralateral retina. Thus, the right visual
cortex subserves the left half of the visual field, and vice versa. Visual
information from the macula lutea is conveyed to the posterior part of
area 17, i.e., the area around the occipital pole.
A unilateral lesion of area 17 produces contralateral
hemianopsia; a partial lesion produces quadrantanopsia in the part of
the visual field that corresponds to the site of the lesion. Central vision is
unimpaired as long as the lesion spares the posterior end of the calcarine
fissure at the occipital pole.
Primary Auditory Cortex
Localization. The primary auditory cortex is located in the
transverse gyri of Heschl (area 41), which form part of the upper surface
of the superior temporal gyrus (Figs. 9.17, 9.18). It receives its afferent
input from the medial geniculate body, which, in turn, receives auditory
impulses from both organs of Corti byway of the lateral lemnisci. Thus,
the primary auditory cortex of each side processes impulses arising in
both ears (bilateral projection).
Unilateral lesions of the primary auditory cortex cause only
subtle hearing loss because of the bilateral projections in the auditory
pathway. The impairment mainly concerns directed hearing, and the
ability to distinguish simple from complex sounds of the same frequency
and intensity.
Primary Gustatory Cortex
Taste-related impulses are processed first in the rostral nucleus of
the tractus solitarius in the brainstem and then conducted, by way of the
central tegmental tract, to a relay station in the ventral posteromedial
nucleus of the thalamus (parvocellular part). They then travel onward
through the posterior limb of the internal capsule to the primary
gustatory cortex, which is located in the pars opercularis of the inferior
frontal gyrus, ventral to the somatosensory cortex and above the lateral
sulcus (area 43, Fig. 9.18).
Primary Vestibular Cortex
Neurons of the vestibular nuclei in the brainstem project
bilaterally to the ventralposterolateral and posteroinferior nuclei of the
thalamus, as well as to its posterior nuclear group near the lateral
geniculate body. Vestibular impulses are conducted from these sites to
area 2v in the parietal lobe, which lies at the base of the intraparietal
sulcus, directly posterior to the hand and mouth areas of the postcentral
gyrus. Electrical stimulation of area 2v in humans induces a sensation of
movement and vertigo. Area 2v neurons are excited by head movement.
They receive visual and proprioceptive as well as vestibular input.
Another cortical area receiving vestibular input is area 3a, at the base of
the central sulcus adjacent to the motor cortex. The function of area 3a
neurons is probably to integrate somatosensory, special sensory, and
motor information for the control of head and body position.
Large lesions of area 2v in humans can impair spatial
orientation.
Association Areas
Unimodal Association Areas
The unimodal association areas of the cortex are located next to
the primary cortical areas. Their function, in very general terms, is to
provide an initial interpretation of the sensory impulses that are
processed in relatively raw form in the primary cortical areas. Sensory
information transmitted to the association areas is compared with
previously stored information, so that a meaning can be assigned to it.
The visual association areas are areas 18 and 19 (Fig. 9.18), which are
adjacent to the primary visual cortex (area 17). These areas receive
relatively basic visual information from area 17 and use it to perform a
higher-level analysis of the visualworld. The somatosensory association
cortex lies just behind the primary somatosensory cortex in area 5, and
the auditory association cortex is part of the superior temporal gyrus
(area 22) (Fig. 9.18). The unimodal association areas receive their neural
input through association fibers from the corresponding primary cortical
fields. They receive no direct input from the thalamus.
Multimodal Association Areas
Unlike the unimodal association areas, the multimodal
association areas are not tightly linked to any single primary cortical
field. They make afferent and efferent connections with many different
areas of the brain and process information from multiple somatosensory
and special sensory modalities (Fig. 9.26).
They are the areas in which motor and linguistic concepts are
first drafted, and in which neural representations are formed that do not
directly depend on sensory input. The largest multimodal association
area is the multimodal portion of the frontal lobe (to be described
further below), accounting for 20% of the entire neocortex. Another
important multimodal association area is found in the posterior portion
of the parietal lobe. While the anterior portion of the parietal lobe
processes somatosensory information (areas 1, 2, 3, and 5), its posterior
portion integrates somatosensory with visual information to enable the
performance of complex movements.
Frontal Lobe
The frontal lobe can be divided into three major components: the
primary motor cortex (area 4), which has already been described, the
premotor cortex (area 6), and the prefrontal region, a large expanse of
cortex consisting of multimodal association areas (Fig. 9.18). The
primary motor cortex and the premotor cortex form a functional system
for the planning and control of movement. The prefrontal cortex is
primarily concerned with cognitive tasks and the control of behavior.
Premotor cortex. The premotor cortex (area 6) is a higher-order
center for the planning and selection of motor programs, which are then
executed by the primary motor cortex. Just as the unimodal association
areas adjacent to the primary somatosensory, visual, and auditory
cortices are thought to store sensory impressions, so too the premotor
cortex is thought to store learned motor processes, acting in cooperation
with the cerebellum and basal ganglia. The stored “motor engrams” can
be called up again for use as needed. Even tasks performed with a single
hand activate the premotor cortex of both hemispheres. Another
important function of the premotor cortex is the planning and initiation
of eye movements by the frontal eye fields (area 8; Figs. 9.17, 9.18, and
9.21). Unilateral stimulation of area 8 induces conjugate movement of
both eyes to the opposite side.
Lesions of area 8 that diminish its activity produce conjugate
gaze deviation to the side of the lesion through the preponderant activity
of the contralateral area 8 (deviation conjugate, e. g., in stroke—“the
patient looks toward the lesion”).
Higher Cortical Functions and Their
Impairment by Cortical Lesions
Aphasia
Language is one of the more important and complex activities of
the human brain. In most individuals (ca. 95%), language-related areas
are located in the frontal and temporoparietal association cortices of the
left hemisphere, which is usually contralateral to the dominant (right)
hand. Some important aspects of language, however, including its
emotional (affective) component, are subserved by the right hemisphere.
The major speech centers are in the basal region of the left frontal lobe
(Broca’s area, area 44) and in the posterior portion of the temporal lobe
at its junction with the parietal lobe (Wernicke’s area, area 22) (Fig.
9.26). These areas are spatially distinct from the primary sensory and
motor cortical areas responsible for purely auditory perception (auditory
cortex, transverse gyri of Heschl), purely visual perception (visual
cortex), and the motor performance of the act of speaking (primary
motor cortex). Broca’s area is activated when an individual speaks, and
even during “silent speech,” i.e., when words and sentences are
formulated without actually being spoken. Pure word repetition, on the
other hand, is associated with activation in the insula. Wernicke’s area is
primarily concerned with the analysis of heard sounds that are classified
as words.
Aphasia. A disturbance of language function is called aphasia
(different subtypes of aphasia are sometimes collectively termed “the
aphasias”). Some types of aphasia exclusively affect speech, writing
(dysgraphia or agraphia), or reading (dyslexia or alexia). Aphasia is
distinct from impairment of the physical act of speaking, which is called
dysarthria or anarthria (caused, for example, by lesions of the pyramidal
tract, cerebellar fiber pathways, the brainstem motor neurons innervating
the muscles of speech, e. g., in bulbar paralysis, or the muscles
themselves).
Dysarthria and anarthria affect articulation and phonation, i.e.,
speech, rather than language production per se (grammar, morphology,
syntax, etc.).
Aphasia is called fluent or nonfluent, depending on whether the
patient speaks easily and rapidly, or only hesitantly and with abnormal
effort. The more important types of aphasia, their distinguishing features,
and their cortical localization are summarized in Table 9.1.
Global aphasia involves all aspects of language and severely
impairs spoken communication. The patient cannot speak spontaneously
or can only do so with great effort, producing no more than fragments of
words. Speech comprehension is usually absent; at best, patients may
recognize a few words, including their own name. Perseveration
(persistent repetition of a single word/subject) and neologisms are
prominent, and the ability to repeat heardwords is markedly impaired.
Patients have great difficulty naming objects, reading, writing, and
copying letters or words. Their ability to name objects, read, and write,
except for the ability to copy letters of the alphabet or isolated words, is
greatly impaired. Language automatism (repetition of gibberish) is a
characteristic feature. Site of lesion: Entire distribution of the middle
cerebral artery, including both Broca’s and Wernicke’s areas.
Broca’s aphasia (also called anterior, motor, or expressive
aphasia) is characterized by the absence or severe impairment of
spontaneous speech, while comprehension is only mildly impaired. The
patient can speak only with great effort, producing only faltering,
nonfluent, garbled words. Phonemic paraphasic errors are made, and
sentences are of simple construction, often with isolated words that are
not grammatically linked (agrammatism, “telegraphic” speech). Naming,
repetition, reading out loud, and writing are also impaired. Site of lesion:
Broca area; may be due to infarction in the distribution of the prerolandic
artery (artery of the precentral sulcus).
Wernicke’s aphasia (also called posterior, sensory, or receptive
aphasia) is characterized by severe impairment of comprehension.
Spontaneous speech remains fluent and normally paced, but
paragrammatism, paraphasia, and neologisms make the patient’s speech
partially or totally incomprehensible (word salad, jargon aphasia).
Naming, repetition of heard words, reading, and writing are also
markedly impaired. Site of lesion: Wernicke’s area (area 22). May be due
to infarction in the distribution of the posterior temporal artery.
Transcortical aphasia. Heard words can be repeated, but other
linguistic functions are impaired: spontaneous speech in transcortical
motor aphasia (syndrome similar to Broca’s aphasia), language
comprehension in transcortical sensory aphasia (syndrome similar to
Wernicke’s aphasia). Site of lesion: Motor type, left frontal lobe
bordering on Broca’s area; sensory type, left temporo-occipital junction
dorsal to Wernicke’s area. Watershed infarction is the most common
cause.
Amnestic (anomic) aphasia. This type of aphasia is
characterized by impaired naming and word finding. Spontaneous speech
is fluent but permeated with word-finding difficulty and paraphrasing.
The ability to repeat, comprehend, and write words is essentially normal.
Site of lesion: Temporoparietal cortex or subcortical white matter.
Conduction aphasia. Repetition is severely impaired; fluent,
spontaneous speech is interrupted by pauses to search for words and by
phonemic paraphasia. Language comprehension is only mildly impaired.
Site of lesion: Arcuate fasciculus or insular region.
Agraphia
Agraphia. Agraphia is the acquired inability to write. Agraphia
may be isolated (due to a lesion located in area 6, the superior parietal
lobule, or elsewhere) or accompanied by other disturbances: aphasic
agraphia is fluent or nonfluent, depending on the accompanying aphasia;
apraxic agraphia is due to a lesion of the dominant parietal lobe; spatial
agraphia, in which the patient has difficulty writing on a line and only
writes on the right side of the paper, is due to a lesion of the
nondominant parietal lobe; alexia with agraphia may be seen in the
absence of aphasia. Micrographia (abnormally small handwriting) is
found in Parkinson disease and is not pathogenetically related to
agraphia. Various forms of agraphia are common in Alzheimer disease.
Examination: The patient is asked to write sentences, long words, or
series of numbers to dictation, to spell words, and to copy written words.
Alexia
Alexia. Alexia is the acquired inability to read. In isolated alexia
(alexia without agraphia), the patient cannot recognize entire words or
read them quickly, but can decipher them letter by letter, and can
understand verbally spelled words. The ability to write is unaffected. The
responsible lesion is typically in the left temporooccipital region with
involvement of the visual pathway and of callosal fibers. Anterior alexia
(difficulty and errors in reading aloud; impaired ability to write, spell,
and copy words) is usually associated with Broca’s aphasia. Central
alexia (combination of alexia and agraphia) is usually accompanied by
right-left disorientation, finger agnosia, agraphia, and acalculia
(Gerstmann syndrome; lesions of the angular and supramarginal gyri), or
by Wernicke’s aphasia. Other features include the inability to understand
written language or to spell, write, or copy words. Examination: The
patient is asked to read aloud and to read individual words, letters, and
numbers; the understanding of spelled words and instructions is tested.
Acalculia
Acalculia. Acalculia is an acquired inability to use numbers or
perform simple arithmetical calculations. Patients have difficulty
counting change, using a thermometer, or filling out a check. Lesions of
various types may cause acalculia. Examination: The patient is asked to
perform simple arithmetical calculations and to read numbers.
Apraxia
Apraxia, in general, is a complex disturbance of voluntary
movement that does not result from weakness or other dysfunction of the
primary motor areas, or from the patient’s lack of motivation or failure to
comprehend the task. It manifests itself as an inability to combine
individual, elementary movements into complex movement sequences,
or to assemble these sequences themselves into still higher-order motor
behaviors. The individual movements themselves, however, can still be
carried out.
Motor apraxia. A patient with severe motor apraxia cannot
execute basic sequences of movements, such as reaching out and
grasping an object, even though isolated testing of the individual muscle
groups involved reveals no weakness in the arm or hand.
Ideomotor apraxia results from lesions of the language-dominant
(left) hemisphere, either in the motor association areas or in the
association and commissural fibers by which they are innervated and
interconnected. A typical clinical finding is the omission, or premature
termination, of individual components of a sequence of movements.
Individual components can also be unnecessarily repeated (motor
perseveration), so that they start at inappropriate times and thereby
impede or interrupt the course of the next movement. Patients with motor
apraxia whose lesions lie in the parietal lobe cannot correctly imitate the
examiner’s movements (e. g., a military salute). These patients can often
still copy facial expressions, while patients with left frontal lobe lesions
can copy complex arm movements, but not facial expressions.
Ideational apraxia. In this rarer type of apraxia, a temporoparietal
lesion in the language-dominant (left) hemisphere impairs the planning
and initiation of complex motor activities. The patient remains able, in
principle, to carry out a complex sequence of movements, but seems not
to comprehend its meaning or purpose. The patient either fails to initiate
the movement or terminates it prematurely.
Construction apraxia. Patients with construction apraxia have
difficulty drawing spatial constructions such as geometrical figures or
objects. This disturbance usually results from a lesion in the parietal lobe
of the non-languagedominant (right) hemisphere.
Most apraxic patients are also aphasic. Patients can suffer from
ideomotor, ideational, and constructive apraxia simultaneously,
depending on the site and extent of the lesion.
Agnosia and Neglect
The anterior portion of the parietal lobe processes somatosensory
signals, while its posterior portion and the visual association cortices are
concerned with the integration of somatosensory, visual, and motor
information. Complex activities, such as pouring a drink while carrying
on a conversation, require the simultaneous integration of many different
perceptual and motor processes: the objects handled (glass, bottle) must
be recognized, which requires conjugate eye movements and visual
processing; reaching, grasping, and pouring movements must be
smoothly executed; and, at the same time, language must be heard,
understood, formulated, and spoken. In order to perform these tasks, the
brain needs internal representations of the body, information about the
positions of the limbs, and a conception of the outside world. These
representations must, in turn, be linked to incoming visual and auditory
signals, and to the brain’s plans for intended movement. The association
cortices and the posterior portion of the parietal lobe play an essential
role in these complex integrative processes. As an illustration of this role,
the posterior portion of the parietal lobe is activated not only by intended
grasping movements induced by visual stimuli, but also by palpation of
an unseen object.
Lesions of the visual association cortices and the parietal lobe can
produce many different types of agnosia, i.e., complex disturbances of
perception. A patient with agnosia cannot recognize objects or
spatiotemporal contexts despite intact primary perception (normal vision,
hearing, and somatic sensation) and motor function (absence of
weakness). Agnosia can be visual, auditory, somatosensory, or spatial.
Visual object agnosia. If the visual association areas are
damaged, the patient can still comprehend the spatial structure of
familiar objects, but can no longer identify them. A bottle, for example,
can be correctly drawn, but cannot be identified as a bottle. Other, more
complex types of visual agnosia include prosopagnosia (the inability to
recognize faces) and alexia (the inability to read).
Somatosensory agnosias. Astereognosia is the inability to
recognize an object by touch alone, even though sensation is intact and
objects can otherwise be named without difficulty. Asomatognosia is a
generally diminished, or even absent, ability to perceive one’s own body.
Gerstmann syndrome consists of the inability to name one’s own
fingers (finger agnosia) along with an impairment of writing (dysgraphia
or agraphia), calculation (dyscalculia or acalculia), and the ability to
distinguish right from left. Gerstmann first described these findings in
1924 in a patient with an ischemic stroke in the territory of the middle
cerebral artery affecting the left parietal lobe.
Neglect. Patients sometimes pay less attention to the side of the
body or visual field opposite a cortical lesion, or ignore it altogether; this
is called neglect. There is often an accompanying unawareness of the
deficit (anosognosia). Neglect usually involves vision, hearing, somatic
sensation, spatial perception, and movement simultaneously. The
causative lesion is usually in the parietal lobe of the non-languagedominant (right) hemisphere. A patient with motor neglect moves one
side of the body very little, or not at all, even though it is not paralyzed.
Normal and Impaired Control of Behavior, Including Social
Behavior
Prefrontal cortex. Cognition and the control of behavior are the
main functions of the multimodal association areas in the frontal lobe
that constitute the prefrontal cortex (Fig. 9.18). Experimental electrical
stimulation of the prefrontal cortex does not induce any motor response.
This portion of the frontal lobe is extraordinarily enlarged in primates,
and particularly in humans; thus, it has long been presumed to be the seat
of higher mental functioning. The frontal cortical fields make reciprocal
connections with the medial nucleus of the thalamus, through which they
receive input from the hypothalamus. They also make very extensive
connections with all other areas of the cerebral cortex. The task of the
prefrontal cortex is the rapid storage and analysis of objective and
temporal information. The dorsolateral prefrontal cortex plays an
essential role in the planning and control of behavior, and the orbital
prefrontal cortex does the same in the planning and control of sexual
behavior.
Lesions of the prefrontal convexity. Patients with bilateral
prefrontal lesions can barely concentrate on a task and are extremely
easy to distract with any new stimulus. They can carry out complex tasks
only in part, or not at all. They have no sense of advance planning and
take no account of future events or of possible problems in the execution
of a task. They often stick rigidly to an idea and fail to adapt to changing
circumstances. In extreme cases, they manifest perseveration, i.e., they
perform the same task again and again, always with the same mistakes.
This deficit is strikingly brought out by the Wisconsin Card Sorting Test,
in which the patient sorts cards bearing various symbols and colors
according to some criterion (e. g., shape), after seeing the examiner do
so. Performance in the first round is usually relatively normal. The
examiner confirms the patient’s success, then changes the sorting
criterion (e. g., to color) without explicitly saying so. A patient with a
prefrontal lesion realizes about as rapidly as a normal individual that the
task has changed, yet keeps sorting according to the old criterion, despite
being immediately informed of each mistake.
Markedly reduced drive and lack of spontaneity are also
characteristic clinical signs of prefrontal dysfunction. These deficits are
revealed by very poor performance on the Word Fluency Test, in which
the patient is given a short period of time to say as manywords as
possible that begin with a particular letter of the alphabet. Patients with
prefrontal lesions do badly despite relatively normal verbal memory.
They do badly on nonverbal tests as well: normal subjects can draw
about 35 pictures in five minutes, patients with left frontal lesions 24,
patients with right frontal lesions 15. Because they lack spontaneity in all
forms of communication, these patients seem lazy, lethargic, and
unmotivated. They neglect many activities of daily life, spend the
morning in bed, fail to wash or groom themselves or to get dressed
without help, and do no regular work. Nonetheless, their formal IQ and
long-term memory are largely intact!
Fronto-orbital lesions. Social and sexual behavior are controlled
and regulated by highly complex processes. Behavior of these types, too,
is abnormal in patients with frontal lobe lesions. Fronto-orbital lesions,
in particular, produce two characteristic types of personality disturbance.
Pseudo-depressive patients are apathetic and indifferent and display
markedly reduced drive, diminished sexual desire, and little or no
variation in their emotional state. Pseudo-psychopathic patients, on the
other hand, are hypomanic and restless in their movements, fail to keep
an appropriate distance from others, and lack normal kinds of inhibition.
They display markedly increased drive and sexual desire. They are
unwilling or unable to hold to the same normal conventions of behavior
that they followed unquestioningly before becoming ill.
Consciousness
Consciousness is an active processwith multiple individual
components, including wakefulness, arousal, perception of oneself and
the environment, attention, memory, motivation, speech, mood,
abstract/logical thinking, and goaldirected action.
Clinical assessment of consciousness tests the patients’
perception of themselves and their environment, behavior, and responses
to external stimuli. Findings are expressed in terms of three categories:
level of consciousness (state/clarity of consciousness, quantitative level
of consciousness, vigilance, alertness, arousability); content of
consciousness (quality of consciousness, awareness); and wakefulness.
Changes in any of these categories tend to affect the others aswell.
Morphologically, the level of consciousness is associated with the
reticular activating system (RAS). This network is found along the entire
length of the brain stem reticular formation, from the medulla to the
intralaminar nuclei of the thalamus. The RAS has extensive bilateral
projections to the cerebral cortex; the cortex also projects back to the
RAS. Neurotransmission in these systems is predominantly with
acetylcholine, monoamines (norepinephrine, dopamine, serotonin),
GABA (inhibitory), and glutamate (excitatory).
In the normal state of consciousness, the individual is fully
conscious, oriented, and awake. All of these categories undergo circadian
variation (depending on the time of day, a person may be fully awake or
drowsy, more or less concentrated, with organized or disorganized
thinking), but normal consciousness with full wakefulness can always be
restored by a vigorous stimulus.
Acute Disturbances of Consciousness
Confusion affects the content of consciousness - attention,
concentration, thought, memory, spatiotemporal orientation, and
perception (lack of recognition). It may also be associated with changes
in the level of consciousness (fluctuation between agitation and
somnolence) and in wakefulness (impaired sleep–wake cycle with
nocturnal agitation and daytime somnolence).
Delirium is characterized by visual hallucinations, restlessness,
suggestibility, and autonomic disturbances (tachycardia, blood pressure
fluctuations, hyperhidrosis).
Somnolence is a mild reduction of the level of consciousness
(drowsiness, reduced spontaneous movement, psychomotor sluggishness,
and delayed response to verbal stimuli) while the patient remains
arousable: he or she is easily awakened by a stimulus, but falls back
asleep once it is removed. The patient responds to noxious stimuli with
direct and goal-directed defensive behavior. Orientation and attention
aremildly impaired but improve on stimulation.
Stupor is a significant reduction of the level of consciousness.
These patients require vigorous and repeated stimulation before they
open their eyes and look at the examiner. They answer questions slowly
and inadequately, or not at all. They may lie motionless or display
restless or stereotyped movements. Confusion reflects concomitant
impairment of the content of consciousness
Disorders of arousal. Wakefulness normally follows a circadian
rhythm. Sleep apnea syndrome, narcolepsy, and parasomnia are disorders
of arousal. Hypersomnia is caused by bilateral paramedian thalamic
infarcts, tumors in the third ventricular region, and lesions of the
midbrain tegmentum. The level and content of consciousness may also
be affected. In patients with bilateral paramedian thalamic infarction, for
example, there may be a sudden onset of confusion, followed by
somnolence and coma. After recovery from the acute phase, these
patients are apathetic and their memory is impaired (“thalamic
dementia”).
Coma (from the Greek for “deep sleep”) is a state of
unconsciousness in which the individual lies motionless, with eyes
closed, and cannot be aroused even by vigorous stimulation. Coma
reflects a loss of the structural or functional integrity of the RAS or the
areas to which it projects. Coma may be produced by an extensive brain
stem lesion or by extensive bihemispheric cerebral lesions, as well as by
metabolic, hypoxic/ischemic, toxic, or endocrine disturbances. In the
syndrome of transtentorial herniation, a large unihemispheric lesion can
cause coma by compressing the midbrain and the diencephalic RAS.
Even without herniation, however, large unihemispheric lesions can
transiently impair consciousness.
Coma Staging
The degree of impairment of consciousness is correlated with the
extent of the causative lesion. The severity and prognosis of coma are
judged from the patient’s response to stimuli. There is no universally
accepted grading system for coma. Proper documentation involves an
exact description of the stimuli given and the responses elicited, rather
than isolated items of information such as “somnolent” or “GCS 10.”
Coma scales (e. g., the Glasgow Coma Scale) are useful for the
standardization of data for statistical purposes but do not replace a
detailed documentation of the state of consciousness.
Spontaneous movement. Assessment of motor function yields
clues to the site of the lesion and the etiology of coma. The examiner
should note the pattern of breathing, any utterances, yawning,
swallowing, coughing, and movements of the limbs (twitching of the
face or hands may indicate epileptic activity; there may be myoclonus or
flexion/extension movements).
Stimuli. Lesions of the midbrain or lower diencephalon produce
the decerebration syndrome (arm/leg extension with adduction and
internal rotation of the arms, pronation and flexion of the hands), while
extensive bilateral lesions at higher levels produce the decortication
syndrome (arm/hand flexion, arm supination, leg extension). These
pathological flexion and extension movements occur spontaneously or in
response to external stimuli (verbal stimulation, tickling around the nose,
pressure on the knuckles or other bones) whether the cause of coma is
structural or metabolic. Withdrawal of the limb from the stimulus usually
means that the pyramidal pathway for the affected limb is intact.
Stereotyped flexion or extension movements are usually seen in patients
with severe damage to the pyramidal tract.
Brainstem reflexes. Structural lesions of the brain stem usually
impair the function of the internal and external eye muscles, while
supratentorial lesions generally do not, unless they secondarily affect the
brain stem. Coma in a patient with intact brainstemreflexes is likely to be
due to severe bihemispheric dysfunction (if no further objective deficit is
found, coma may be psychogenic or factitious). Physicians should be
aware that coma due to intoxication or drug overdose may be difficult to
distinguish from that due to structural damage by clinical examination
alone. Preservation of the vestibulo-ocular reflex (VOR) and of the doll’s
eyes reflex is compatible with either a bihemispheric lesion or a toxic or
metabolic disorder. The VOR induces conjugate eye movement only if
its brain stem pathway is intact (from the cervical spinal cord to the
oculomotor nucleus). Nonetheless, the VOR may be absent in some
cases of toxic coma (due to, e. g., alcohol, barbiturates, ).
Abnormalities of the respiratory pattern are of limited
localizing value. Cheyne–Stokes respiration is characterized by regular
waxing and waning of the tidal volume, punctuated by apneic pauses. It
has a number of causes, including bihemispheric lesions and metabolic
disorders. Slow, shallow respiration usually reflects a metabolic or toxic
disorder. Rapid, deep respiration (Kussmaul’s respiration) usually
reflects a pontine or mid brain lesion, or metabolic acidosis. Medullary
lesions and extensive supratentorial damage produce ataxic, cluster, or
gasping respiration.